Autoimmune Pancreatitis

1 What is Autoimmune Pancreatitis?

As the name suggests, autoimmune pancreatitis (AIP) is a chronic condition of pancreatitis caused by autoimmune condition.

In this condition, the immune system attacks the tissues of pancreas.

This form of chronic pancreatitis responds to steroid therapy in controlling the symptoms.

There are two types of AIP – Type 1 (IgG4-related pancreatitis) and Type 2.

Type 1 AIP is a part of another condition called IgG4-related disease (IgG4-RD).

This disease affects many organs including

  • pancreas,
  • liver,
  • kidneys,
  • lymph nodes.

Type 2, on the other hand, affects only the pancreas, and most of the patients have associated inflammatory bowel disease.

AIP is rare and is often misdiagnosed as pancreatic cancer. Differential diagnosis of the condition is essential as AIP responds to steroid treatment. 

2 Symptoms

Autoimmune Pancreatitis may remain asymptomatic in most of the cases. Signs and symptoms, when present, resemble that of pancreatic cancer.

Common symptoms of the condition include

  • Dark-colored urine
  • Pale stools
  • Jaundice
  • Pain in the upper part of the abdomen
  • Back pain
  • Nausea and vomiting
  • Weakness and fatigue
  • Loss of appetite
  • Unexplained weight loss

Blockage of the bile ducts cause jaundice, and this is the most common symptom of AIP. Autoimmune condition may cause formation of masses in the pancreas which is often misunderstood for tumor or cancer.

Type AIP is more commonly seen in males over 60 years. Many organs like

  • bile ducts,
  • salivary glands,
  • lungs

may be involved in the condition.

Type 1 is also characterized by the presence of fibrous tissue behind the stomach and intestine, a condition called as retroperitoneal fibrosis (RPF).

is also seen associated with this type of AIP.

Type 2 AIP is more commonly seen in people over 40 years, but is seen equally among males and females. Most of them have increased risk of inflammatory bowel disease like ulcerative colitis.

3 Causes

As in many other conditions, Autoimmune pancreatitis is also caused by the autoimmune condition in which the immune system attacks pancreatic tissue.

The actual cause of autoimmune condition is not known. 

4 Making a Diagnosis

Signs and symptoms of Autoimmune pancreatitis is similar to that of pancreatic cancer, making diagnosis hard.

Misdiagnosis cause delay in treatment of the condition.

As per the International Consensus Diagnostic Criteria (ICDC), the diagnostic tests for AIP are

  • Histology
  • Imaging techniques like CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP)
  • Serology
  • Other organ involvement
  • Response to steroid trial – a trial of response to steroids is used to confirm the diagnosis, as only AIP responds to steroid treatment

Blood test checks for the presence of immunoglobulin IgG4.

Elevated levels of these immunoglobulins indicate type 1 AIP.

Biopsy of pancreatic tissue shows the distinctive cells and tissues of the organ in AIP.

5 Treatment

Several treatment methods exist for Autoimmune pancreatitis.

Draining the biliary ducts to remove obstruction in the ducts helps to control the symptoms.

This is done by inserting a tube into the ducts by a process called biliary stenting.

Drainage is suggested even when diagnosis is not confirmed.

Prednisone treatment often given immediate response to symptoms.

Corticosteroids like prednisone may have long term side effects.

To reduce the side effects, immunosuppressants or immunomodulators like

  • mycophenolate,
  • mercaptopurine,
  • azathioprine

are recommended.

Pancreatic enzyme supplementation is often needed when enzymes are less.

Type 1 AIP is associated multiple organ involvement like

  • lymph glands,
  • salivary glands,
  • liver.

Continuous monitoring for organ involvement is also recommended in the treatment of AIP. 

6 Prevention

There is no standard preventive measure for Autoimmune pancreatitis, as the actual cause of the disease is not known.

Understanding and avoiding risk factors help to control the condition. 

7 Alternative and Homeopathic Remedies

A few alternative and homeopathic remedies exist for Autoimmune pancreatitis.

Dietary changes and supplements are the natural remedies used in AIP.

Anti-inflammatory compounds like aloe and papaya juice are used in pancreatitis treatment.

These supplements control inflammation and offer relief from symptoms.

  • Dandelion root,
  • licorice,
  • cramp bark

are also suggested.

Antioxidants are useful in reducing pain and improving health. 

8 Lifestyle and Coping

Understanding the condition and the treatment strategies help in coping with Autoimmune pancreatitis.

Following a healthy diet helps to improve overall health of the patient.

Supplements should be taken only after recommendation from the doctors.

Corticosteroid therapy may cause several side effects.

9 Risk and Complications

Complications associated with Autoimmune pancreatitis are:

  • Pancreatic insufficiency,
  • diabetes,
  • pancreatic stone formation

Long-term use of steroids cause side effects. 

10 Related Clinical Trials